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Updated: Feb 4, 2026

The Murine Choline-Deficient, Ethionine-Supplemented CDE Diet Model of Chronic Liver Injury
Published on: October 21, 2017
Alpha-1-Antitrypsin Deficiency Liver Disease
Dhiren Patel1, Jeffrey H Teckman2
1Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Saint Louis University School of Medicine, 1465 South Grand Boulevard, St Louis, MO 63104, USA.
Abstract:
In homozygous ZZ alpha-1-antitrypsin (AAT) deficiency, the liver synthesizes large quantities of AAT mutant Z, which folds improperly during biogenesis and is retained within the hepatocytes and directed into intracellular proteolysis pathways. These intracellular polymers trigger an injury cascade, which can lead to liver injury. This is highly variable and not all patients develop liver disease. Although not fully described, there is likely a strong influence of genetic and environmental modifiers of the injury cascade and of the fibrotic response. With improved understanding of liver injury mechanisms, new strategies for treatment are now being explored.
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